seen in connective tissue diseases, including:
scleroderma ("progressive systemic sclerosis", PSS)
The CREST form of scleroderma tends to be confined to the skin of the fingers and face.
The skin changes and other features of disease tend to occur more slowly than in the diffuse form.
A characteristic clinical pattern can occur in patients with the CREST form of scleroderma.
This name represents the following features:[see above ]
C.....Calcinosis, refers to the formation of tiny deposits of calcium in the skin.
This is seen as hard whitish areas in the superficial skin,
These firm deposits can be tender, can become infected, and can fall off spontaneously or require surgical removal.
R.....Raynauds phenomenon refers to the spasm of the tiny artery vessels supplying blood to
These areas turns blue, white, then red after exposure to extremes of cold, or even sometimes with extremes of heat or emotional upset.
E.....Esophagus disease in scleroderma is characterized by poorly functioning muscle of the lower 2/3 of the esophagus.
This can lead to an abnormally wide esophagus which allows stomach acid to backflow into the esophagus to cause heartburn, inflammation, and potentially scarring.
This can eventually lead to difficulty in passing food from the mouth through the esophagus into the stomach[dysphagia].
Symptoms of heartburn are treated aggressively in patients with scleroderma in order to prevent injury to the esophagus.
S.....Sclerodactyly refers to the localized thickening and tightness of the skin of the fingers or toes.
This can give them a "shiny" appearance.
The tightness can cause severe limitation of motion of the fingers and toes.
These skin changes generally progress much slower that those of patients with the diffuse form of scleroderma.
T.....Telangiectasia are tiny red areas,
and in the mouth behind the lips.
These areas blanch when they are pressed upon and represent dilated capillaries.
Patients can have variations of CREST
, i.e. CRST, REST, ST, etc. Patients can also have "overlap" illness with features of both CREST
and the diffuse form of scleroderma.
Some patients have overlaps of scleroderma and other connective tissue diseases, such as
systemic lupus erythematosus,
Approximately 10 percent of patients with the CREST
variant develop elevated pressures in the blood vessels to the lungs (pulmonary hypertension).
It takes only two of the five CREST symptoms for a diagnosis of CREST (either “pure” or “plus”) to be made.
For example, a patient with Calcinosis and Raynaud’s would have CREST (which for precision may also be written as Crest, but it is CREST nonetheless.)
a kind of classification according to the criterias found:
“Pure”CREST — by itself: “Pure” CREST is diagnosed when patients have two or more symptoms of CREST but they do not meet the criteria for either Limited or Diffuse Scleroderma.
That is, they must not have tight skin above their wrists, and if there is tight skin on their fingers, they must not have either pitting digital ulcers or lung fibrosis
"Plus” CREST — along with: When CREST symptoms appear along with another form of Scleroderma, it is referred to as, for example, “Limited Scleroderma plus CREST” or “Diffuse Scleroderma plus CREST.” A person may also have any other autoimmune disease “plus CREST.”
Progression of CREST
Although some doctors still believe CREST is a subcategory, the existing research studies have been unable to predict consistently how (or whether) the disease will progress to Diffuse Systemic Scleroderma in any specific individual.